Gene Symbol: blw
Description: bellwether
Alias: A2(2nd)27, ATP syn-alpha, ATP5a, ATPA, ATPA_DROME, ATPsyn-alpha, ATPsynalpha, Blw, C-V-alpha, C-Va, CG3612, CValpha, CxV, Dmel\CG3612, l(2)01410, l(2)03972, l(2)59ABa, l(2)k00212, ms(2)01504, ms(2)Pry58F, bellwether, ATP SYNTHASE ALPHA, ATP synthase, ATP synthase alpha chain, ATP synthase alpha subunit of complex V, ATP synthase subunit alpha, ATP synthase subunits alpha, ATP synthase, alpha subunit, ATP synthase, subunit alpha, ATP synthetase alpha chain, CG3612-PA, COMPLEX V: alpha chain, F0/F1 ATP synthase alpha, alpha subunit of ATP synthase, alpha subunit of the mitochondrial F[[1]]F[[0]] ATPase, alpha-ATP synthase, alpha-subunit of ATP synthase, bellweather, blw-PA, colibri, complex V alpha, complex valpha, complex valpha subunit, mitochondrial ATP synthase alpha subunit, mitochondrial ATP synthase subunit B, mitochondrial ATP synthase subunit alpha of complex V, mitochondrial ATPase complex V-subunit alpha
Species: fruit fly
Products:     blw

Top Publications

  1. Pimenta de Castro I, Costa A, Lam D, Tufi R, Fedele V, Moisoi N, et al. Genetic analysis of mitochondrial protein misfolding in Drosophila melanogaster. Cell Death Differ. 2012;19:1308-16 pubmed publisher
    ..We show that in flies, a pathway involving pink1, parkin and ref(2)P has a role in the maintenance of a viable pool of cellular mitochondria by promoting organellar quality control. ..
  2. Ziviani E, Tao R, Whitworth A. Drosophila parkin requires PINK1 for mitochondrial translocation and ubiquitinates mitofusin. Proc Natl Acad Sci U S A. 2010;107:5018-23 pubmed publisher
    ..We hypothesize that Mfn ubiquitination may provide a mechanism by which terminally damaged mitochondria are labeled and sequestered for degradation by autophagy. ..
  3. Li Y, Fetchko M, Lai Z, Baker N. Scabrous and Gp150 are endosomal proteins that regulate Notch activity. Development. 2003;130:2819-27 pubmed
    ..Gp150 concentrated Sca protein in late endosomes. A model is proposed in which endosomal Sca and Gp150 promote Notch activation in response to Delta, by regulating acquisition of insensitivity to Delta in a subset of cells. ..
  4. Devorkin L, Go N, Hou Y, Moradian A, Morin G, Gorski S. The Drosophila effector caspase Dcp-1 regulates mitochondrial dynamics and autophagic flux via SesB. J Cell Biol. 2014;205:477-92 pubmed publisher
    ..These data reveal a new mitochondrial-associated molecular link between nonapoptotic caspase function and autophagy regulation in vivo. ..
  5. Tufi R, Gandhi S, de Castro I, Lehmann S, Angelova P, Dinsdale D, et al. Enhancing nucleotide metabolism protects against mitochondrial dysfunction and neurodegeneration in a PINK1 model of Parkinson's disease. Nat Cell Biol. 2014;16:157-66 pubmed publisher
    ..We propose that targeting strategies enhancing nucleotide synthesis pathways may reverse mitochondrial dysfunction and rescue neurodegeneration in PD and, potentially, other diseases linked to mitochondrial impairment. ..
  6. Zeng Q, Avilov V, Shippy S. Prefractionation methods for individual adult fruit fly hemolymph proteomic analysis. J Chromatogr B Analyt Technol Biomed Life Sci. 2016;1015-1016:74-81 pubmed publisher
    ..The demonstration of this method establishes a means to deepen proteomic analysis to this commonly used, important biological model system. ..
  7. Galloni M, Edgar B. Cell-autonomous and non-autonomous growth-defective mutants of Drosophila melanogaster. Development. 1999;126:2365-75 pubmed
    ..The isolation of non-cell-autonomous larval growth-defective mutants suggests that specialized organs coordinate growth throughout the animal and provides new tools for studies of organismal growth regulation. ..
  8. Shim M, Kim J, Jung H, Lee K, Xu X, Carlson B, et al. Elevation of glutamine level by selenophosphate synthetase 1 knockdown induces megamitochondrial formation in Drosophila cells. J Biol Chem. 2009;284:32881-94 pubmed publisher
  9. Lindsay H, Baines R, ffrench Constant R, Lilley K, Jacobs H, O Dell K. The dominant cold-sensitive Out-cold mutants of Drosophila melanogaster have novel missense mutations in the voltage-gated sodium channel gene paralytic. Genetics. 2008;180:873-84 pubmed publisher
    ..Therefore, in the absence of suitable vertebrate models, Ocd provides a system in which genetic, molecular, physiological, and behavioral tools can be exploited to determine mechanisms underlying sodium channel periodic paralyses...

More Information


  1. Ozdowski E, Mowery Y, Cronmiller C. Stall encodes an ADAMTS metalloprotease and interacts genetically with Delta in Drosophila ovarian follicle formation. Genetics. 2009;183:1027-40 pubmed publisher
  2. Celotto A, Chiu W, Van Voorhies W, Palladino M. Modes of metabolic compensation during mitochondrial disease using the Drosophila model of ATP6 dysfunction. PLoS ONE. 2011;6:e25823 pubmed publisher
    ..ATP6(1)animals have a nearly complete loss of ATP synthase activity and an acute bioenergetic deficit when they are asymptomatic, but surprisingly we discovered no ..
  3. DUBOFF B, Götz J, Feany M. Tau promotes neurodegeneration via DRP1 mislocalization in vivo. Neuron. 2012;75:618-32 pubmed publisher
    ..Our results thus identify actin-mediated disruption of mitochondrial dynamics as a direct mechanism of tau toxicity in neurons in vivo...
  4. Tiefenböck S, Baltzer C, Egli N, Frei C. The Drosophila PGC-1 homologue Spargel coordinates mitochondrial activity to insulin signalling. EMBO J. 2010;29:171-83 pubmed publisher
    ..Taken together, our data place Spargel at a nodal point for the integration of mitochondrial activity to tissue and organismal metabolism and growth. ..
  5. Terhzaz S, Southall T, Lilley K, Kean L, Allan A, Davies S, et al. Differential gel electrophoresis and transgenic mitochondrial calcium reporters demonstrate spatiotemporal filtering in calcium control of mitochondria. J Biol Chem. 2006;281:18849-58 pubmed
    ..The results highlight the dynamic nature and both spatial and temporal heterogeneity of calcium signaling possible in differentiated, organotypic cells and provide a new model for neuroendocrine control of V-ATPase. ..
  6. Scialò F, Sriram A, Fernández Ayala D, Gubina N, Lõhmus M, Nelson G, et al. Mitochondrial ROS Produced via Reverse Electron Transport Extend Animal Lifespan. Cell Metab. 2016;23:725-34 pubmed publisher
    ..These results illustrate that the source of a ROS signal is vital in determining its effects on cellular physiology and establish that manipulation of ubiquinone redox state is a valid strategy to delay aging. ..
  7. Backes S, Hess S, Boos F, Woellhaf M, Gödel S, Jung M, et al. Tom70 enhances mitochondrial preprotein import efficiency by binding to internal targeting sequences. J Cell Biol. 2018;217:1369-1382 pubmed publisher
  8. Sieber M, Thomsen M, Spradling A. Electron Transport Chain Remodeling by GSK3 during Oogenesis Connects Nutrient State to Reproduction. Cell. 2016;164:420-32 pubmed publisher
    ..Our studies reveal an important link between metabolism and oocyte maturation. ..
  9. de Castro I, Costa A, Celardo I, Tufi R, Dinsdale D, Loh S, et al. Drosophila ref(2)P is required for the parkin-mediated suppression of mitochondrial dysfunction in pink1 mutants. Cell Death Dis. 2013;4:e873 pubmed publisher
    ..We conclude that Ref(2)P is a crucial downstream effector of a pathway involving Pink1 and Parkin and is responsible for the maintenance of a viable pool of cellular mitochondria by promoting their aggregation and autophagic clearance. ..
  10. Murphy K, Tabuloc C, Cervantes K, Chiu J. Ingestion of genetically modified yeast symbiont reduces fitness of an insect pest via RNA interference. Sci Rep. 2016;6:22587 pubmed publisher
    ..To our knowledge, this is the first study to show that yeast can be used to deliver dsRNA to an insect pest. ..
  11. Humphrey D, Parsons R, Ludlow Z, Riemensperger T, Esposito G, Verstreken P, et al. Alternative oxidase rescues mitochondria-mediated dopaminergic cell loss in Drosophila. Hum Mol Genet. 2012;21:2698-712 pubmed publisher
  12. Knoblich J, Lehner C. Synergistic action of Drosophila cyclins A and B during the G2-M transition. EMBO J. 1993;12:65-74 pubmed
    ..However, mitotic spindles are clearly abnormal and progression through mitosis is delayed in these cyclin B deficient embryos. ..
  13. Liu W, Acin Perez R, Geghman K, Manfredi G, Lu B, Li C. Pink1 regulates the oxidative phosphorylation machinery via mitochondrial fission. Proc Natl Acad Sci U S A. 2011;108:12920-4 pubmed publisher
    ..Taken together, these results suggest a unique pathogenic mechanism of PINK1 PD: The loss of PINK1 impairs mitochondrial fission, which causes defective assembly of the ETC complexes, leading to abnormal bioenergetics. ..
  14. Khanna M, Stanley B, Thomas G. Towards a membrane proteome in Drosophila: a method for the isolation of plasma membrane. BMC Genomics. 2010;11:302 pubmed publisher
    ..This points the way for further adaptations into other systems. ..
  15. McQuibban A, Joza N, Megighian A, Scorzeto M, Zanini D, Reipert S, et al. A Drosophila mutant of LETM1, a candidate gene for seizures in Wolf-Hirschhorn syndrome. Hum Mol Genet. 2010;19:987-1000 pubmed publisher
    ..Taken together our results demonstrate the function of DmLETM1 as a mitochondrial osmoregulator through its KHE activity and uncover a pathophysiological WHS phenotype in the model organism D. melanogaster. ..
  16. Talamillo A, Chisholm A, Garesse R, Jacobs H. Expression of the nuclear gene encoding mitochondrial ATP synthase subunit alpha in early development of Drosophila and sea urchin. Mol Biol Rep. 1998;25:87-94 pubmed
    Complementary DNAs encoding nuclear-coded mitochondrial ATP synthase subunit alpha of Drosophila melanogaster and Strongylocentrotus purpuratus were obtained by a combination of library screening and redundant PCR...
  17. Santaren J, Van Damme J, Puype M, Vandekerckhove J, Garcia Bellido A. Identification of Drosophila wing imaginal disc proteins by two-dimensional gel analysis and microsequencing. Exp Cell Res. 1993;206:220-6 pubmed
    ..As an illustration we present 12 of them: 8 corresponding to proteins already known in Drosophila and the 4 showing homologies with proteins of other organisms. ..
  18. Sen A, Damm V, Cox R. Drosophila clueless is highly expressed in larval neuroblasts, affects mitochondrial localization and suppresses mitochondrial oxidative damage. PLoS ONE. 2013;8:e54283 pubmed publisher
    ..Thus Clu's role in mitochondrial function is not critical during larval development, but is important for pupae and adults. ..
  19. Baltzer C, Tiefenböck S, Marti M, Frei C. Nutrition controls mitochondrial biogenesis in the Drosophila adipose tissue through Delg and cyclin D/Cdk4. PLoS ONE. 2009;4:e6935 pubmed publisher
    ..These findings are the first genetic evidence that the regulation of mitochondrial mass can be uncoupled from mitochondrial activity. ..
  20. Sun Y, An S, Henrich V, Sun X, Song Q. Proteomic identification of PKC-mediated expression of 20E-induced protein in Drosophila melanogaster. J Proteome Res. 2007;6:4478-88 pubmed publisher
    ..The data further indicate that PKC-mediated phosphorylation is also required for genes regulated indirectly by 20E-induced changes in the larval salivary gland...
  21. Teixeira F, Sanchez C, Hurd T, Seifert J, Czech B, Preall J, et al. ATP synthase promotes germ cell differentiation independent of oxidative phosphorylation. Nat Cell Biol. 2015;17:689-96 pubmed publisher
    ..genes required for germline stem cell differentiation in the Drosophila ovary, we find that the mitochondrial ATP synthase plays a critical role in this process...
  22. Otani T, Oshima K, Onishi S, Takeda M, Shinmyozu K, Yonemura S, et al. IKK? regulates cell elongation through recycling endosome shuttling. Dev Cell. 2011;20:219-32 pubmed publisher
    ..Our results establish an evolutionarily conserved function of IKK-related kinases in regulating recycling endosome dynamics and point to a key role of endosome dynamics in cell morphogenesis. ..
  23. Di Cara F, Duca E, Dunbar D, Cagney G, Heck M. Invadolysin, a conserved lipid-droplet-associated metalloproteinase, is required for mitochondrial function in Drosophila. J Cell Sci. 2013;126:4769-81 pubmed publisher
    ..Here, we present data demonstrating that invadolysin interacts physically with three mitochondrial ATP synthase subunits...
  24. Sing A, Tsatskis Y, Fabian L, Hester I, Rosenfeld R, Serricchio M, et al. The atypical cadherin fat directly regulates mitochondrial function and metabolic state. Cell. 2014;158:1293-1308 pubmed publisher
    ..Defects in mitochondrial activity in ft mutants are independent of Hippo and PCP signaling and are reminiscent of the Warburg effect. ..
  25. Talamillo A, Fernández Moreno M, Martinez Azorin F, Bornstein B, Ochoa P, Garesse R. Expression of the Drosophila melanogaster ATP synthase alpha subunit gene is regulated by a transcriptional element containing GAF and Adf-1 binding sites. Eur J Biochem. 2004;271:4003-13 pubmed
    ..performed a detailed analysis of the promoter region of the alpha subunit of the Drosophila melanogaster F1F0 ATP synthase complex...
  26. Jacobs H, Stratmann R, Lehner C. A screen for lethal mutations in the chromosomal region 59AB suggests that bellwether encodes the alpha subunit of the mitochondrial ATP synthase in Drosophila melanogaster. Mol Gen Genet. 1998;259:383-7 pubmed
    ..molecular characterization of the corresponding insertion sites, suggests that the previously described male sterile mutation bellwether is an allele of an essential gene that encodes the alpha subunit of the mitochondrial ATP synthase.
  27. Cooley L, Berg C, Spradling A. Controlling P element insertional mutagenesis. Trends Genet. 1988;4:254-8 pubmed